Malignant congenital tumor of the pineal gland rudiment. Literature review and clinical case; Злокачественная врожденная опухоль зачатка шишковидной железы. Обзор литературы и клинический случай

We present a clinical case of an extremely rare congenital brain tumor in a 6-month-old child – pineal anlage tumor (PAT), which occurs in 0.04 % of all brain neoplasms in childhood. Foreign literature describes 20 cases of PAT in children, while there are no such publications in the domestic literature. At present, this tumor is not yet defined as a separate type in the latest edition of the WHO classification in 2021. In the WHO classification of2007, PATis describedas a rare variantof pineoblastoma withmelanotic, cartilaginous and/or rhabdomyoblastdifferentiation, characterized by heterogeneous elements of neuroepithelial and ectomesenchymal tissue, but without endodermal structures. Due to the rarity of this type of neoplasm, histologic diagnosis can be difficult for the morphologist. Specific therapy has not been developed at present, the optimal treatment is the maximal complete surgical removal of the volumetric neoplasm, as well as polychemotherapy according to the protocol of pineoblastoma treatment. However, the efficacy of this strategy remains unclear. © 2025 Elsevier B.V., All rights reserved.